Flashcards in Lung Deck (452):
0
Congenital pulmonary-airway malformation: Definition.
Malformation of part of the lung parenchyma, with abnormal direct connections to the normal tracheobronchial tree.
1
Congenital pulmonary-airway malformation: Blood supply.
Derived from the pulmonary circulation.
2
Congenital pulmonary-airway malformation, type 0: Gross pathology.
Lung parenchyma appears solid.
3
Congenital pulmonary-airway malformation, type 0: Histology (2).
Disorganized proximal airways; no distal airways.
Intervening tissue consists of connective tissue, thick-walled arteries, and extramedullary hematopoiesis.
4
Congenital pulmonary-airway malformation, type 1: Gross pathology.
Medium and large interconnecting cysts, usually limited to one lobe.
5
Congenital pulmonary-airway malformation, type 1: Histology.
Cysts are lined by bronchial epithelium and often contain bands of smooth muscle.
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Congenital pulmonary-airway malformation, type 2: Gross pathology.
Back-to-back cysts, smaller than the cysts of type 1.
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Congenital pulmonary-airway malformation, type 2: Histology.
Cysts are separated by alveolar ductlike structures, blood vessels, skeletal muscle.
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Congenital pulmonary-airway malformation, type 2: Associations.
Sirenomelia.
Renal malformations.
Diaphragmatic hernia.
Cardiovascular abnormalities.
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Congenital pulmonary-airway malformation, type 3: Gross pathology.
Solid mass involving the lobe or the entire lung, with hypoplasia of the other lung.
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Congenital pulmonary-airway malformation, type 3: Histology.
Glandlike structures lined by low cuboidal epithelium.
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Congenital pulmonary-airway malformation, type 3: Associations.
Male sex.
Polyhydramnios.
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Congenital pulmonary-airway malformation, type 4: Gross pathology.
Peripherally located cysts of variable size.
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Congenital pulmonary-airway malformation, type 4: Histology.
Cysts can have thick walls and are lined by a single layer of pneumocytes.
Capillary beds beneath the epithelial lining.
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Congenital pulmonary-airway malformation, type 4: IHC.
Pneumocytes express TTF-1 and surfactant proteins A and B.
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Congenital pulmonary-airway malformation: Limitations on diagnosis.
Diagnosis cannot be made when there is chronic inflammation and fibrosis.
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Bronchopulmonary sequestration: Definition (2).
Anomalous segment of lung has no connection with the normal tracheobronchial tree.
Blood supply comes from the systemic circulation.
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Bronchopulmonary sequestration: Types (2).
Intralobar.
Extralobar.
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Bronchopulmonary sequestration, intralobar type: Typical location.
Lower lobe.
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Bronchopulmonary sequestration, intralobar type: Gross pathology (3).
No pleural covering.
Sharply demarcated from the normal lung tissue.
May have vascular pedicle.
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Bronchopulmonary sequestration, intralobar type: Histology (3).
Marked chronic inflammation.
Mucus accumulation and microcyst formation.
Dense fibrosis surrounds remnants of bronchioles.
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Bronchopulmonary sequestration, extralobar type: Typical location.
Left side.
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Bronchopulmonary sequestration, extralobar type: Gross pathology (2).
Covered with visceral pleura.
Separate from normal lung tissue.
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Bronchopulmonary sequestration, extralobar type: Histology (3).
Enlarged bronchi, bronchioles, alveoli.
No significant inflammation or fibrosis.
Dilated subpleural lymphatics.
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Bronchopulmonary sequestration, extralobar type: Association.
Congenital pulmonary-airway malformation, type 2, is present in up to half of cases.
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Bronchogenic cyst: Origin.
Anomalous budding of the tracheobronchial anlage during development.
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Bronchogenic cyst: Most common site.
Mediastinum.
No communication with the tracheobronchial tree.
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Bronchogenic cyst: Radiography.
May have an air-fluid level.
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Bronchogenic cyst: Histology (3).
Epithelial lining and wall (smooth muscle, cartilage) resemble those of a normal bronchus.
Squamous metaplasia or chronic inflammation may occur.
No alveolar tissue.
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Congenital lobar emphysema: Definition.
Hyperinflation of one or more lobes of the lung.
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Congenital lobar emphysema:
A. Age at presentation.
B. Most common site.
A. Within 6 months after delivery.
B. Left upper lobe.
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Congenital lobar emphysema: Radiography.
Compression of contralateral lung and mediastinal shift.
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Congenital lobar emphysema: Histology.
Alveolar distention without fibrosis.
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Congenital lobar emphysema: Etiologies.
Idiopathic: Most cases.
Intrinsic compression of the bronchus supplying the affected lobe: Structural defect of bronchus.
Extrinsic compression: Mass.
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Chronic bronchitis: Histology.
Reid index >0.5 (glands make up more than half the thickness of the bronchial wall).
Mild chronic inflammation.
35
Asthma: Gross pathology (3).
Hyperinflated lungs.
Mucous plugging of airways.
Saccular bronchiectasis.
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Asthma: Histology (3).
Mucous plugs containing eosinophils.
Fibrosis beneath the basement membrane with patchy loss of epithelium.
Thickened walls of airways due to edema, hyperplasia of smooth muscle, increased mucous glands.
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Asthma vs. chronic bronchitis.
Chronic bronchitis: Few or no eosinophils.
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Bronchiectasis: Definition.
The bronchus is wider than its bronchial artery.
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Bronchiectasis: Clinical presentation.
Persistent cough.
Copious, foul-smelling sputum.
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Bronchiectasis: Causes (8).
Primary ciliary dyskinesia.
Rheumatoid arthritis.
Immunodeficiency.
Cystic fibrosis.
Inflammatory bowel disease.
No known cause (30%).
Graft-versus-host disease.
Infections.
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Bronchiectasis: Histology (2).
Mucosa: Variable inflammation, reactive changes, necrosis.
Wall: Chronic inflammation, fibrosis.
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Middle-lobe syndrome:
A. Definition.
B. Causes.
A. Recurrent or persistent atelectasis of the right middle lobe.
B. Lymphadenopathy, malignancy.
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Type of emphysema associated with
A. Cigarette smokers (2).
B. α₁-Antitrypsin deficiency.
A. Proximal acinar or centrilobular.
B. Panacinar or panlobular.
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Types of emphysema associated with bullous disease and idiopathic spontaneous pneumothorax (2).
Distal acinar, paraseptal.
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Emphysema: Histology (2).
Alveolar distention without fibrosis.
Club-shaped septa may project into the alveolar spaces.
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Interstitial emphysema.
Presence of air outside the airways, e.g. in the connective tissue and around bronchovascular bundles.
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Constrictive bronchiolitis: Site.
Terminal conducting airways.
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Constrictive bronchiolitis: Associations (8).
Collagen-vascular disease.
Hypersensitivity pneumonitis.
Inhalational injury.
Neuroendocrine hyperplasia/tumors.
Viral infection.
Organ transplantation.
Inflammatory bowel disease.
Drugs.
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Constrictive bronchiolitis: Histology (4).
Fibrosis.
Chronic inflammation with epithelial metaplasia.
Smooth-muscle hyperplasia.
Luminal obliteration.
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Acute bronchiolitis:
A. Age group.
B. Associations (2).
A. Infants and children.
B. Viral infection, bacterial infection.
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Acute bronchiolitis: Histology (3).
Intense acute and chronic inflammation of small bronchioles.
Epithelial necrosis and sloughing.
Edema.
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Diffuse bronchiolitis:
A. Epidemiology.
B. Associations (4).
A. Affects Asian adults.
B. HLA Bw54; cold agglutinin, increased ESR, leukocytosis.
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Diffuse bronchiolitis: Histology (3).
Lymphocytes, plasma cells, foamy macrophages.
Many intraluminal neutrophils.
Organization of exudate to form polypoid plugs.
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Respiratory bronchiolitis: Association.
Cigarette smoking.
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Respiratory bronchiolitis: Histology (4).
Interstitial inflammation.
Many intraluminal pigmented macrophages.
Smooth-muscle hypertrophy.
Mild fibrosis.
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Mineral-dust bronchiolitis: Type of lung disease.
Restrictive, due to fibrosis.
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Mineral-dust bronchiolitis: Histology (3).
Fibrosis.
Deposits of dust mainly around respiratory bronchioles.
Luminal narrowing.
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Follicular bronchiolitis: Type of lung disease.
Obstructive, due to external compression.
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Follicular bronchiolitis: Histology.
Lymphoid hyperplasia with reactive germinal centers.
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Follicular bronchiolitis: Associations.
Anything that causes lymphoid hyperplasia, e.g. chronic inflammation, infections.
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Diffuse alveolar damage: Clinical equivalents (3).
Acute respiratory-distress syndrome.
Acute interstitial pneumonia.
Acute lung injury.
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Diffuse alveolar damage: Relevance to autoimmune disease (2).
Various collagen-vascular diseases cause DAD-type inflammation.
Various vasculitides can resemble AIP clinically.
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Diffuse alveolar damage: Distribution of lesions (2).
Patchy involvement of the lung, but concentrated in the lower lobes.
Diffuse involvement of the alveolus.
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Diffuse alveolar damage: Radiography.
Ground-glass opacities that spare the lobules.
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Diffuse alveolar damage: Phases.
Exudative: First week.
Proliferative: Second week.
Fibrotic: Late.
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Diffuse alveolar damage, exudative phase: Histology.
Hyaline membranes and interstitial edema.
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Diffuse alveolar damage, proliferative phase: Histology.
Interstitium and airspaces: Florid proliferation of fibroblasts, myofibroblasts, type 2 pneumocytes.
Arteries: Intimal proliferation, medial hypertrophy.
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Diffuse alveolar damage, fibrotic phase: Histology.
Dense interstitial fibrosis with microcysts.
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Diffuse alveolar damage vs. usual interstitial pneumonia (3).
UIP:
− No hyaline membranes.
− Temporal heterogeneity of fibrosis.
− Fibrosis has more collagen and fewer cells.
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Acute respiratory-distress syndrome: Prognosis.
Most patients regain near-normal lung function.
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Acute interstitial pneumonia:
A. Clinical presentation.
B. Prognosis.
A. Resembles severe community-acquired pneumonia but does not respond to antibiotics.
B. Death in 6 months in 78% of cases.
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Cryptogenic organizing pneumonia:
A. Clinical presentation.
B. Duration.
A. Cough, dyspnea, and flulike symptoms.
B. Subacute.
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Cryptogenic organizing pneumonia: Prognosis.
Usually responds to steroids.
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Cryptogenic organizing pneumonia: Distribution of lesions.
Subpleural.
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Cryptogenic organizing pneumonia: Radiography.
Peribronchial consolidation and nodularity.
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Cryptogenic organizing pneumonia: Histology (2).
Masson bodies: Intraluminal plugs consisting of young fibrous tissue.
Interstitial mild chronic inflammation with foci of foamy macrophages.
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Cryptogenic organizing pneumonia: Special stain.
Movat's stain: Masson bodies appear green; dense fibrosis would appear yellow.
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Cryptogenic organizing pneumonia vs. usual interstitial pneumonia.
UIP:
− Dense fibrosis (not seen in COP).
− Fibroblastic foci are interstitial, not intraluminal.
− Temporal heterogeneity of fibrosis.
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Cryptogenic organizing pneumonia vs. nonspecific interstitial pneumonia.
NSIP: Interstitial chronic inflammation without Masson bodies.
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Usual interstitial pneumonia:
A. Clinical presentation.
B. Duration.
A. Progressive dyspnea and cough.
B. Chronic.
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Usual interstitial pneumonia:
A. Associated environmental agents (3).
B. Associated inherited diseases (2).
A. Cigarettes, asbestos, drugs.
B. Familial idiopathic pulmonary fibrosis, Hermansky-Pudlak syndrome.
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Usual interstitial pneumonia: Association that imparts a better prognosis.
Collagen-vascular disease.
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Usual interstitial pneumonia: Prognosis.
Median survival is 3 years.
84
Usual interstitial pneumonia: Distribution of lesions (2).
Subpleural.
Lower lobes.
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Usual interstitial pneumonia: Radiography (3).
Honeycombing.
Ground-glass opacities.
Traction bronchiectasis.
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Usual interstitial pneumonia: Histology.
Temporal heterogeneity: The same area may contain both mature fibrosis and subepithelial young fibrosis (fibroblastic foci).
Spatial heterogeneity: Some areas are affected, some not.
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Usual interstitial pneumonia: Histologic findings associated with poor prognosis (4).
Diffuse alveolar damage.
Infection.
Capillaritis.
Organizing pneumonia.
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Usual interstitial pneumonia vs. hypersensitivity pneumonitis with fibrosis.
Hypersensitivity pneumonitis:
− Mainly upper lobes.
− Centered on bronchioles.
− More cellular inflammation, including giant cells or poorly formed granulomas.
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Usual interstitial pneumonia vs. Langerhans' cell histiocytosis.
Langerhans' cell histiocytosis:
− Centered on bronchioles.
− Few or no fibroblastic foci.
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Usual interstitial pneumonia: How to diagnose cases that contain findings of other interstitial lung diseases.
As usual interstitial pneumonia.
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Nonspecific interstitial pneumonia:
A. Clinical presentation.
B. Duration.
A. Dyspnea, cough, fever.
B. Subacute.
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Nonspecific interstitial pneumonia: Associations (5).
Cigarettes.
Drugs.
Immunodeficiency.
Collagen-vascular diseases.
Hypersensitivity pneumonitis.
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Nonspecific interstitial pneumonia: Distribution of lesions.
Subpleural.
Lower lobes.
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Nonspecific interstitial pneumonia: Radiography.
Peribronchial ground-glass opacities.
Reticular opacities.
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Nonspecific interstitial pneumonia: Long-term prognosis.
Cellular type: Excellent.
Fibrotic type: Poor.
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Nonspecific interstitial pneumonia, cellular type: Histology (3).
Diffuse interstitial infiltrate of lymphocytes and plasma cells.
Preservation of the pulmonary architecture.
Hyperplasia of type 2 pneumocytes.
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Nonspecific interstitial pneumonia, fibrotic type: Histology (4).
Loose to dense interstitial fibrosis that thickens the alveolar walls.
Fibrosis shows temporal homogeneity.
Preservation of the pulmonary architecture.
Mild or moderate chronic inflammation.
98
Nonspecific interstitial pneumonia: Histological clue to an association.
Abundance of lymphoid aggregates suggests collagen-vascular disease.
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Nonspecific interstitial pneumonia: What should not be seen histologically (3).
Significant honeycombing.
Many fibroblastic foci.
Granulomas.
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Nonspecific interstitial pneumonia vs. lymphoid interstitial pneumonia.
LIP:
− Denser inflammatory infiltrate.
− Architectural distortion.
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Desquamative interstitial pneumonia: Clinical presentation.
Dyspnea, cough, chest pain.
102
Desquamative interstitial pneumonia: Duration.
Subacute.
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Desquamative interstitial pneumonia: Distribution of lesions.
Subpleural.
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Desquamative interstitial pneumonia:
A. Frequent association.
B. Infrequent associations (2).
A. Cigarette smoking, even years after cessation.
B. Sirolimus; mutations in SP-C, the gene for surfactant protein C.
105
Desquamative interstitial pneumonia: Radiography.
Ground-glass opacities.
Thin-walled cysts.
Reticular opacities.
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Desquamative interstitial pneumonia: Histology (3).
Pigmented macrophages (originally thought to be "desquamated" pneumocytes) fill alveoli.
Intraalveolar laminated basophilic concretions ("blue bodies") sometimes present.
No significant fibrosis.
107
Desquamative interstitial pneumonia: Special stain.
Prussian blue highlights the finely granular pigment within the macrophages.
108
Desquamative interstitial pneumonia vs. respiratory bronchiolitis-interstitial lung disease.
RB-ILD: Macrophages fill the bronchioles but not the distal airspaces.
109
Lymphoid interstitial pneumonia: Associations.
Children: AIDS.
Adults: Immunocompromise, including AIDS.
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Lymphoid interstitial pneumonia: Clinical presentation in children.
Manifestations of AIDS: Recurrent infections, parotiditis, failure to thrive.
Respiratory failure occasionally.
111
Lymphoid interstitial pneumonia: Radiography (2).
Children: Miliary reticulonodular infiltrates.
Adults: The same plus alveolar consolidation.
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Lymphoid interstitial pneumonia: Histology (2).
Dense mononuclear inflammation of the interstitium, sometimes with germinal centers.
Partial obliteration of architecture.
113
Lymphoid interstitial pneumonia: Microbiological association.
ISH detects EBV in most cases.
114
Lymphoid interstitial pneumonia: Clinical course.
No progression to fibrosis.
115
Hypersensitivity pneumonitis:
A. Associations.
B. Distribution of lesions.
A. Various organic antigens, esp. those of thermophilic actinomycetes and birds.
B. Upper lobes, peribronchiolar.
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Hypersensitivity pneumonitis: Phases (3).
Acute: Exposure to high concentrations of antigen; onset in 4-8 hours; resolution in 24-48 hours.
Subacute: Continuous or intermittent exposure to low concentrations of antigen; responds to steroids or to withdrawal of antigen.
Chronic: Subacute plus fibrosis; worse prognosis.
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Hypersensitivity pneumonitis, subacute phase: Histology (2).
Small, poorly formed granulomas and mononuclear inflammation next to bronchioles.
Foamy histiocytes in alveoli and interstitium.
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Hypersensitivity pneumonitis, chronic phase: Histologic patterns (3).
NSIP-like pattern: Homogeneous fibrosis with architectural preservation.
UIP-like: Patchy subpleural fibrosis with architectural distortion.
Irregular peribronchiolar pattern: UIP-like plus peribronchiolar fibrosis.
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Hypersensitivity pneumonitis vs. usual interstitial pneumonia.
UIP:
− No giant cells, no granulomas.
− Mainly subpleural and in lower lobes.
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Hypersensitivity pneumonitis vs. nonspecific interstitial pneumonia.
NSIP: No giant cells, no granulomas.
Clinical history may be required to make the distinction.
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Hypersensitivity pneumonitis vs. sarcoidosis.
Sarcoidosis:
− Well-formed granulomas with hyalinized rim and location along bronchovascular bundles.
− No UIP- or NSIP-like changes.
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Eosinophilic lung diseases: Unknown etiology (3)
Simple eosinophilic pneumonia.
Acute eosinophilic pneumonia.
Chronic eosinophilic pneumonia.
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Eosinophilic lung diseases: Known etiology (4).
Allergic bronchopulmonary aspergillosis.
Bronchocentric granulomatosis.
Parasitic infections.
Drugs.
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Eosinophilic lung diseases: Vasculitic causes (2).
Allergic angiitis.
Churg-Strauss syndrome.
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Eosinophilic lung disease: How to diagnose without tissue or cytology.
Demonstrate pulmonary opacities and peripheral eosinophilia.
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Acute eosinophilic pneumonia:
A. Clinical presentation.
B. Associations (2).
A. Acute respiratory distress that mimics infectious pneumonia.
B. Cigarettes, dust.
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Acute eosinophilic pneumonia: Histology (3).
Resembles acute phase of DAD but with alveolar and interstitial eosinophils.
Hypertrophied and detached type 2 pneumocytes.
Intact basal lamina.
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Acute eosinophilic pneumonia: Prognosis.
Rapid and complete response to corticosteroids.
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Acute eosinophilic pneumonia: Degree of eosinophilia.
BAL: More than 25%.
Peripheral blood: Often no eosinophilia at first.
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Chronic eosinophilic pneumonia: Radiography.
Peripheral consolidation mainly involving middle and lower zones.
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Chronic eosinophilic pneumonia: Laboratory abnormalities (2).
Peripheral eosinophilia.
Elevated IgE in 7% of patients.
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Chronic eosinophilic pneumonia: Histology.
Intraalveolar and interstitial eosinophils (single or in aggregates) and eosinophilic giant cells.
Damage to basal lamina, leading to fibrosis.
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Parasites that can cause eosinophilic lung disease: Allergic reaction (3).
Entamoeba.
Toxocara.
Clonorchis sinensis.
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Parasites that can cause eosinophilic lung disease: Direct invasion (4).
Ascaris lumbricoides.
Ankylostoma duodenale.
Paragonimus westermani.
Schistosomes.
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Parasites that can cause eosinophilic lung disease: Others (3).
Strongyloides stercoralis.
Microfilariae.
Dirofilaria immitis.
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Sarcoidosis: Frequency of pulmonary disease.
90-95%.
137
Pulmonary sarcoidosis: Clinical course (2).
Abrupt, acute illness with a better prognosis.
Chronic, insidious illness with persistent, progressive course.
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Pulmonary sarcoidosis: Distribution of lesions (3).
Around the lymphatic vessels in the pleura, the interlobular septa, and the bronchovascular bundles.
139
Histology of pulmonary sarcoidosis:
A. Periphery of granulomas.
B. Tissues involved by granulomas.
A. Concentric fibrosis often; usually no cuff of lymphocytes.
B. Vessels, pleura.
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Histology of pulmonary sarcoidosis: Inclusions that can be confused for microorganisms (2).
Hamazaki-Wesenberg bodies: GMS (+), AFB (+); mimic fungi.
Microcalcifications: Mimic fungi or P. jiroveci.
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Sarcoidosis vs. hypersensitivity pneumonitis.
Hypersensitivity pneumonitis:
− Granulomas are less well formed.
− More inflammation in the interstitium.
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Types of disease associated with a sarcoidosis-like disorder (2).
Malignancies.
Collagen-vascular disorders.
143
Idiopathic pulmonary hemosiderosis: Age group.
Children and adolescents.
144
Idiopathic pulmonary hemosiderosis:
A. Clinical manifestations (4).
B. Clinical course.
A. Cough, hemoptysis, iron-deficiency anemia, weight loss.
B. Subject to spontaneous remission or exacerbation.
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Idiopathic pulmonary hemosiderosis: Associations (3).
IgA nephropathy.
Dermatitis herpetiformis.
Celiac disease.
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Idiopathic pulmonary hemosiderosis: Gross pathology.
Heavy, red-brown lung tissue.
147
Idiopathic pulmonary hemosiderosis: Histology (2).
Intraalveolar dense groups of hemosiderin-laden macrophages, or frank hemorrhage.
Loss or hyperplasia of alveolar epithelium.
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Idiopathic pulmonary hemosiderosis: Pertinent negative findings (5).
Granulomas.
Vasculitis.
Infarction.
Infection.
Immune complexes or immunoglobulins.
149
Idiopathic pulmonary hemosiderosis: Treatment.
Immunosuppression.
150
Goodpasture's syndrome: Relevance of epidemiology to presentation (2).
Young white males: Pulmonary symptoms often precede renal symptoms.
Elderly women: Glomerulonephritis and renal failure precede pulmonary disease.
151
Goodpasture's syndrome: Pulmonary manifestation.
Hemoptysis, which may be mild or life-threatening.
152
Goodpasture's syndrome: Histology of pulmonary disease (3).
Intraalveolar hemorrhage with many hemosiderin-laden macrophages.
Fibrous thickening of alveolar septa.
Hyperplasia of pneumocytes.
153
Goodpasture's syndrome: Ancillary tests (2).
Immunofluorescence: Linear IgG, IgM, or IgA and complement along the alveolar basement membrane.
Serology: Circulating autoantibodies.
154
Goodpasture's syndrome: Electron microscopy.
Fragmented capillary basement membranes.
Widened gaps between endothelial cells.
155
Goodpasture's syndrome: Antibodies (2).
Anti-GBM against the non-collagenous domain of the α₃ chain of type IV collagen.
Concurrent c-ANCA or p-ANCA in one third of patients.
156
Goodpasture's syndrome: Associated HLA type.
DR2.
157
Pulmonary silicosis: Classification according to timing.
Acute: Symptoms within 3 years after exposure.
Accelerated: Within 3-10 years.
Chronic: At least 20 years.
158
Pulmonary silicosis: Classification according to gross pathology.
Simple: Nodules up to 1 cm.
Progressive massive: Nodules >1 cm.
159
Pulmonary silicosis, acute: Histology (3).
Pulmonary edema.
Interstitial inflammation.
PAS-positive granular substance fills alveoli.
160
Pulmonary silicosis, chronic: Histology.
Discrete fibrous nodules of variable size, mainly in the upper lobes and subpleural regions.
161
Pulmonary silicosis, chronic: Structure of nodules.
Center: Amorphous.
Middle zone: Layers of dense collagen with focal calcification and necrosis.
Periphery: Particle-laden macrophages, lymphocytes, fibroblasts.
162
Microbiological association of pulmonary silicosis:
A. Organism.
B. Histology.
A. Mycobacterium tuberculosis (silicotuberculosis).
B. Silicotic nodules with central necrosis and epithelioid granulomas.
163
Pulmonary silicosis vs. pulmonary alveolar proteinosis.
Pulmonary alveolar proteinosis:
− Little inflammation or fibrosis.
− Protein is reactive for antibodies to surfactant apoptotein.
164
Pulmonary alveolar proteinosis: Associations (3).
Inorganic dust.
Hematological malignancy.
Immunodeficiency.
165
Pulmonary alveolar proteinosis: Complication.
Secondary infection by
− Fungi.
− Viruses.
− Pneumocystis jiroveci.
− Nocardia.
− Mycobacteria.
166
Pneumoconiosis: Particles that induce fibrosis (5).
Silica.
Coal dust.
Asbestos.
Beryllium.
Talc.
167
Pneumoconiosis: Particles that induce little or no fibrosis (3).
Iron oxide.
Tin.
Barium.
168
Asbestosis:
A. Timing.
B. Clinical presentation (3).
A. Symptoms appear 15-20 years after exposure.
B. Dyspnea, clubbing, restrictive lung disease.
169
Asbestosis: Location of lesions.
Mostly in the lower lobes.
170
Asbestosis: How the inhaled fibers get to the pleura.
Through the lymphatic channels (carried in macrophages) or by direct penetration.
171
Asbestos: Chemical composition.
Hydrated magnesium silicates.
172
Asbestosis: Appearance of fibers in tissue (2).
Asbestos body: Brown (due to hemosiderin), beaded, two bulbous ends, clear core.
Ferruginous body: No clear core.
173
Asbestosis: Histology of tissue reaction (3).
Diffuse interstitial fibrosis with chronic inflammation.
Hyperplasia of type 2 pneumocytes.
Alveolar epithelial cells may contain a substance that resembles Mallory's hyaline.
174
Asbestosis: Preferred sample for recovery of asbestos bodies.
Bronchioloalveolar lavage.
175
Asbestosis: Related cancers.
Mesothelioma.
Lung cancer.
176
Radiation pneumonitis, acute:
A. Timing.
B. Clinical presentation.
A. Symptoms begin between 6 weeks and 6 months after exposure.
B. Cough, dyspnea on exertion.
177
Radiation pneumonitis: Exacerbating factors (3).
Chemotherapy.
Prior irradiation.
Infection.
178
Radiation pneumonitis, acute: Histology.
Hyaline membranes as in diffuse alveolar damage.
Interstitial proliferation of atypical fibroblasts within young fibrosis.
179
Radiation pneumonitis, fibrotic stage: Histology.
Resembles NSIP but with hyperplasia and cytologic atypia of type 2 pneumocytes.
180
Radiation pneumonitis: Typical cells (2).
Foam cells: Lipid-rich cells with features of macrophages and smooth-muscle cells.
Radiation fibroblasts: Stromal cells with atypical nuclei and much blue cytoplasm.
181
Radiation pneumonitis: Prognosis (2).
Acute pneumonitis usually responds to corticosteroids.
Carcinoma may arise after >10 years.
182
Bleomycin toxicity:
A. Incidence.
B. Mechanism.
A. Less than 5%.
B. The lungs are relatively deficient in hydrolase, which detoxifies bleomycin.
183
Bleomycin toxicity: Exacerbating factors (3).
Oxygen.
Cyclophosphamide and other drugs.
Radiation.
184
Bleomycin toxicity: Radio-recall phenomenon
Bleomycin can unmask damage to lungs caused by previous irradiation.
185
Bleomycin toxicity: Histology.
Diffuse alveolar damage.
Atypia of pneumocytes.
Progression to nonuniform fibrosis in some patients.
186
Amiodarone toxicity: Histology.
Diffuse alveolar damage.
Hyperplasia of type 2 pneumocytes.
Foamy histiocytes in airspaces.
187
Amiodarone toxicity: Electron microscopy.
Lamellar inclusions in alveolar macrophages.
188
Methotrexate toxicity: Histology.
Poorly formed granulomas.
Interstitial inflammation that includes eosinophils.
189
Wegener's granulomatosis: Clinical triad.
Disease of upper airways (e.g. sinusitis).
Disease of lower airways.
Glomerulonephritis.
190
Wegener's granulomatosis: Affected sites (3).
Head and neck.
Lungs.
Kidneys.
191
Wegener's granulomatosis: Autoantibody.
c-ANCA in most: Usually against proteinase 3.
192
Wegener's granulomatosis in the lungs: Distribution of lesions.
Mostly in the lower lobes.
193
Wegener's granulomatosis in the lungs: Gross pathology.
Many nodules of variable size.
Cavitation in half of cases.
194
Wegener's granulomatosis in the lungs: Histology (5).
Geographic necrosis.
Granulomatous inflammation.
Palisades of histiocytes.
Microabscesses.
Vasculitis of small vessels.
195
Wegener's granulomatosis in the lungs: Special stain.
Elastic stain: Destruction of elastic lamina.
196
Wegener's granulomatosis vs. Churg-Strauss syndrome (5).
Churg-Strauss syndrome:
− Eosinophilia in blood and tissues is typical.
− Asthma is typical.
− Cardiac disease is common.
− Renal disease is mild.
− Sinus disease is mild.
197
Churg-Strauss syndrome: Clinical tetrad.
Asthma.
Rhinitis.
Peripheral eosinophilia.
Systemic vasculitis.
198
Churg-Strauss syndrome: Autoantibody.
p-ANCA in some: Usually against myeloperoxidase.
199
Churg-Strauss syndrome in the lungs: Gross pathology (4).
Multifocal consolidation.
Eosinophilic pleural effusion.
Stellate peripheral pulmonary arteries.
Cavitation is rare.
200
Churg-Strauss syndrome in the lungs: Histology (4).
Eosinophilic pneumonia.
Diffuse hemorrhage.
Vasculitis.
Extravascular granulomas with central necrosis.
201
Churg-Strauss syndrome in the lungs: Pharmacological differential diagnosis.
Carbamazepine-induced vasculitis.
202
Churg-Strauss syndrome in the lungs: Microbiological differential diagnoses.
Parasitic infection.
Fungal infection.
203
Pulmonary arterial hypertension: Associations with systemic diseases (4).
Scleroderma.
Sickle-cell disease.
Rheumatoid arthritis.
Systemic lupus erythematosus.
204
Pulmonary hypertension: Symptoms (5).
Dyspnea.
Chest pain.
Syncope.
Cough.
Hemoptysis is rare.
205
Pulmonary hypertension: Leading cause.
Left-sided heart failure.
206
Pulmonary hypertension:
A. Definition.
B. Gross pathology.
A. Pulmonary systolic pressure >25 mmHg.
B. Pulmonary atherosclerosis.
207
Grading scheme for pulmonary hypertension: Use.
Applies only to idiopathic pulmonary hypertension and to certain types of secondary pulmonary hypertension ("APAH").
208
Grading scheme for pulmonary hypertension: Histology of Grade I (2).
Medial hypertrophy of pulmonary arteries.
Extension of muscle into walls of pulmonary arterioles.
209
Grading scheme for pulmonary hypertension: Histology of Grade II.
Grade I plus proliferation of intimal cells.
210
Grading scheme for pulmonary hypertension: Histology of Grade III (3).
Grade I plus subendothelial fibrosis.
Small arteries and arterioles: Concentric masses of fibrous tissue, reduplication of elastic lamina, occlusion of vascular lumens.
Large arteries: Atherosclerosis.
211
Grading scheme for pulmonary hypertension: Histology of Grade IV (3).
Medial hypertrophy is less apparent.
Progressive dilatation of small arteries.
Plexiform lesions.
212
Grading scheme for pulmonary hypertension: Histology of Grade V (2).
Plexiform and angiomatoid lesions.
Intraalveolar hemosiderin-laden macrophages.
213
Grading scheme for pulmonary hypertension: Histology of Grade VI (2).
Necrotizing arteritis with thrombosis.
Transmural infiltrates of neutrophils and eosinophils.
214
Grading scheme for pulmonary hypertension: Which grades are reversible?
Grades I, II, and III.
215
Grading scheme for pulmonary hypertension: Which grades are associated with secondary pulmonary hypertension?
Grades I, II, and III.
216
Pulmonary hypertension due to chronic thrombotic or embolic disease: Histology (3).
Little or no medial hypertrophy.
Eccentric intimal fibrosis with focal obliteration.
Organizing thrombi with recanalization.
217
Pulmonary hypertension: Mutation.
Familial pulmonary arterial hypertension: BMPR4 on 2q33-q34.
218
Pulmonary veno-occlusive disease: Age group.
Mostly in children and young adults.
219
Pulmonary veno-occlusive disease: Distribution of lesions.
Pulmonary venules and small veins in the lobular septa.
220
Pulmonary veno-occlusive disease: Histology of veins.
Occlusion of affected veins by intimal fibrosis.
Medial hypertrophy ("arterialization") and increase in elastic fibers.
Usually no plexiform lesions or vascular inflammation.
221
Pulmonary veno-occlusive disease: Additional histology.
Capillaries may be dilated and tortuous, mimicking pulmonary capillary hemangiomatosis.
Hemosiderin may be abundant, mimicking idiopathic pulmonary hemosiderosis.
222
Pulmonary capillary hemangiomatosis.
Proliferation of capillaries causes thickening of alveolar septa.
223
CMV pneumonia: Infected cells (4).
Fibroblasts.
Endothelial cells.
Respiratory epithelial cells.
Macrophages.
224
CMV pneumonia: Effect of ganciclovir on histology.
Makes the intranuclear inclusions redder and rounder.
225
Herpes simplex virus: Respiratory infections.
Necrotizing tracheobronchitis.
Necrotizing bronchiolocentric pneumonia.
Interstitial pneumonitis resembling DAD.
226
Measles virus: Respiratory infections.
Necrotizing bronchiolitis.
Giant-cell pneumonia.
227
Parainfluenza virus:
A. Cytopathic effects.
B. Respiratory infection.
A. None or multinucleate giant cells.
B. Giant-cell pneumonia (genotypes 2 and 3).
228
Hantavirus:
A. Cytopathic effect.
B. Histology of respiratory infection (2).
A. None; virus is identified by IHC or PCR.
B. Marked alveolar edema; immature leukocytes in alveolar capillaries.
229
Legionella pneumonia: Type of inflammation (3).
Neutrophilic.
Monocytes and macrophages.
All of the above.
230
Legionella pneumonia: Other histologic features (3).
Intraalveolar fibrin and hemorrhage.
Abundant nuclear debris.
Vasculitis occasionally.
231
Legionella pneumonia:
A. Special stain.
B. Other method of detection.
A. Silver stain.
B. Urinary antigen test detects serogroup 1 only.
232
Nocardia pneumonia: Main route of infection.
Inhalation of bacteria in soil and decaying organic matter.
233
Nocardia pneumonia: Sites of concurrent infection (4).
Skin.
Bone.
Kidney.
Brain.
234
Nocardia pneumonia: Histology (3).
Necrosis.
Microabscesses.
Immunocompromised: Poorly formed granulomas rather than abscesses.
235
Nocardia pneumonia: Special stains (3).
Gram stain.
Fite's stain.
Silver stain.
236
Ghon lesion of pulmonary tuberculosis:
A. Size.
B. Consistency.
C. Location.
A. 1-2 cm.
B. Necrotic center.
C. Lower upper lobe or upper lower lobe, near the pleura.
237
Pulmonary tuberculosis: Ghon complex.
Consists of a Ghon lesion and enlarged hilar lymph nodes.
238
Pulmonary tuberculosis: Ranke complex.
Fibrosis and calcification of the Ghon complex due to cell-mediated immunity.
239
Pulmonary tuberculosis: Characteristic cell.
The Langhans cell: A giant cell with peripherally arranged nuclei, formed by the fusion of the histiocytes that surround the granuloma.
240
Pulmonary tuberculosis: Complications of growth of lesion (4).
Cavitation.
Empyema.
Bronchopneumonia.
Embolization.
241
Mycobacterium tuberculosis: Virulence factors (3).
Proteins of the cell envelope.
Lipopolysaccharide.
Mycobacterial cell-entry protein (Mcep).
242
Mycobacterium tuberculosis: Proteins of the cell envelope that impart virulence (3).
Peptidoglycan.
Arabin-galactan.
Mycolic acids.
243
Mycobacterium tuberculosis:
A. Lipopolysaccharide.
B. Gene for the cell-entry protein.
A. Lipoarabinomannan.
B. mce1A.
244
Mycobacterium avium complex: Most likely portal of entry.
The gastrointestinal tract.
245
Atypical mycobacterial infections: Histology (3).
Necrotizing granulomas.
Non-necrotizing granulomas.
Immunocompromised patients: Poorly organized infiltrates of histiocytes, nonspecific inflammation.
246
Pulmonary disease due to rapidly growing mycobacteria: Leading causes (2).
M. abcessus.
M. fortuitum.
247
Hyphae of Aspergillus spp.:
A. Branching.
B. Septa.
A. Dichotomous; acute-angle.
B. Frequent.
248
Hyphae of Fusarium spp.:
A. Branching.
B. Septa.
A. Right-angle mostly.
B. Frequent.
249
Hyphae of Pseudallescheria boydii.:
A. Branching.
B. Septa.
A. Haphazard.
B. Frequent.
250
Hyphae of zygomycetes:
A. Branching.
B. Septa.
C. Differences from other fungal hyphae (2).
A. Haphazard; obtuse-angle.
B. Inconspicuous.
C. Wider (up to 25 μm); non-parallel sides.
251
Zygomycosis: Most common genus.
Rhizopus.
252
Zygomycosis: Portal of entry.
Inhalation of spores.
253
Zygomycosis: Predisposing factors (6).
Breach of skin or mucosa.
Iatrogenic immunosuppression.
Neutropenia.
Diabetes mellitus.
Antibiotics, broad-spectrum.
Severe malnutrition.
254
Zygomycosis: Vascular lesions (2).
Invasion of vessels.
Granulomatous vasculitis.
255
Growth of zygomycetes in tissue:
A. Facilitating enzyme.
B. Role of iron.
A. Ketone reductase permits growth in acidic, glucose-rich environments.
B. Essential for growth; deferroxamine increases susceptibility to zygomycosis.
256
Pulmonary aspergillosis: Patterns of disease (3).
Colonization of old cavity lesion (fungus ball).
Hypersensitivity reactions.
Invasion.
257
Pulmonary aspergillosis: Target lesion.
Necrotic center surrounded by hemorrhagic rim or infarct.
258
Pulmonary aspergillosis: Classic tissue reaction.
Hemorrhagic infarct with sparse inflammation.
259
Bronchocentric granulomatosis: Causes.
Infection.
Noninfectious process such as allergy.
260
Bronchocentric granulomatosis: Histology (2).
Necrotizing granulomatous inflammation destroys small bronchi and bronchioles.
Palisading histiocytes replace the airway walls.
261
Histoplasmosis: Clinical forms (3).
Acute pulmonary histoplasmosis.
Chronic pulmonary histoplasmosis.
Disseminated histoplasmosis.
262
Acute pulmonary histoplasmosis: Clinical presentations (2).
Self-limiting illness in a young child with first exposure to H. capsulatum.
Acute, severe illness similar to ARDS, resulting from exposure to a large inoculum of the fungus.
263
Chronic pulmonary histoplasmosis: Clinical presentation.
Cavitary lesions in an adult with underlying lung disease.
264
Disseminated histoplasmosis: Risk factors (3).
Infancy.
Immunosuppression.
Congenital T-cell deficiency.
265
Histoplasmosis: Histology (3).
Necrotizing granulomas with thick fibrous capsule.
Concentric calcification may impart "tree-bark" appearance.
Immunocompromised: Organisms within foamy macrophages.
266
Pulmonary histoplasmosis: Complications (5).
Granulomatous mediastinitis: Matting and caseous necrosis of mediastinal lymph nodes.
Mediastinal fibrosis: Young adults; often lethal.
Pericarditis, pleural disease, broncholithiasis.
267
Primary pulmonary coccidioidomycosis: Usual clinical presentation.
Asymptomatic or subclinical; self-limited.
268
Pulmonary coccidioidomycosis: Cutaneous manifestations (2).
Erythema nodosum.
Erythema multiforme.
269
Blastomycosis: Initial clinical presentation.
Flulike illness.
270
Blastomycosis: Histology.
Abscesses at first.
Necrotizing granulomas later.
Disseminated infection: "Yeast lakes" with little inflammation.
271
Blastomycosis: Radiography.
Can be indistinguishable from primary pulmonary malignancy.
272
Pulmonary cryptococcosis:
A. Usual clinical presentation.
B. Usual radiography.
A. Asymptomatic.
B. Inapparent.
273
Pulmonary cryptococcosis:
A. Diagnosis (2).
B. Population at risk for severe disease.
A. Culture or cytology of bronchioloalveolar lavage.
B. Immunocompromised.
274
Pulmonary cryptococcosis: Gross pathology.
Focal consolidation with gelatinous cut surface.
275
Pulmonary cryptococcosis: Histopathology in the immunocompetent (2).
Granulomas with fibrosis.
Organisms within giant cells and macrophages.
276
Pulmonary cryptococcosis: Histopathology in the immunocompromised (3).
Organisms may fill alveoli and cause minimal inflammation.
Dense aggregation of fungi may induce a fibrohistiocytic reaction.
Organisms may lack capsules.
277
Pneumocystis jiroveci: Four types of infection.
Asymptomatic.
Infantile pneumonia.
Pneumonia in the immunocompromised.
Extrapulmonary infections.
278
Pneumocystis jiroveci: Risk factors for infantile pneumonia (2).
Malnutrition.
Prematurity.
279
Pneumocystis jiroveci: Sites of extrapulmonary infection (4).
Bone marrow.
Lymph nodes.
Spleen.
Liver.
Others.
280
Pneumocystis jiroveci: Radiography.
CT: Bilateral alveolar and interstitial infiltrates radiating from the hilum.
281
Pneumocystis jiroveci: Life cycle.
Trophozoites conjugate to form cysts, which contain sporozoites, which develop into trophozoites.
282
Pneumocystis jiroveci: Microscopy of cysts in vivo.
Spherical and contain up to 8 sporozoites that measure 1-2 μm.
Empty cysts resemble cups or helmets.
283
Pneumocystis jiroveci: Special stains used in identifying cysts (2).
GMS: Best stain; dots within cysts are not sporozoites.
Toluidine blue.
284
Pneumocystis jiroveci: Special stains used in identifying trophozoites and sporozoites (3).
Wright, Giemsa, and Wright-Giemsa.
285
Pneumocystis jiroveci: Another stain.
Immunofluorescence: Monoclonal antibody against the wall of the cyst.
286
Pneumocystis jiroveci: Targets of PCR (4).
Better: Mitochondrial 23S RNA (mtLSUrRNA), internal transcribed spacers.
Others: Cytoplasmic 5S RNA, dihydrofolate reductase regions.
287
Pneumocystis jiroveci: Peak incidence of pneumonia in children with HIV.
At 3-6 months after delivery.
288
Lung transplantation: Early complications (5).
Acute rejection.
Bacterial infection.
Pulmonary edema.
ARDS.
Diffuse alveolar hemorrhage.
289
Acute cellular rejection: Timing.
Usually between 3 and 6 months, but sometimes as early as 1 week or not until years later.
290
Acute cellular rejection:
A. Incidence.
B. Helpful clinical test.
A. More than 80%.
B. Forced exploratory volume in 1 second (FEV₁) is the most sensitive clinical test.
291
Criteria of an ideal transbronchial biopsy to be examined for transplant rejection.
At least 3-5 fragments.
At least 100 alveoli and 1 bronchiole.
292
Acute rejection of lung transplant: Possibly associated histologic finding.
Lymphocytic bronchitis / lymphocytic bronchiolitis.
293
Chronic rejection of lung transplant:
A. Possibly associated histologic finding.
B. Timing of this finding.
C. Recognition of this finding.
A. Bronchiolitis obliterans (fibrous obliteration).
B. Usually within a year.
C. Pulmonary-function tests; biopsy not required.
294
Grading of rejection of lung transplants: A0.
No infiltrate of leukocytes.
No hemorrhage.
No necrosis.
295
Grading of rejection of lung transplants: A1.
Perivascular lymphoid cuff, at least two cell layers thick.
Infiltrate is hard to detect at low power.
296
Grading of rejection of lung transplants: A2.
Perivascular lymphoid cuff is at least 3 cell layers thick.
No neutrophils, but eosinophils may be seen.
297
Grading of rejection of lung transplants: A3.
Expansion of lymphoid infiltrate into the interstitium.
Neutrophils may be apparent.
Endothelialitis may be seen.
298
Grading of rejection of lung transplants: A4.
Diffuse alveolar damage.
299
Grading of rejection of lung transplants: B0.
No inflammation of airways.
300
Grading of rejection of lung transplants: B1.
Minimal inflammation of the airways.
301
Grading of rejection of lung transplants: B2.
Cuff of mononuclear cells, with occasional eosinophils, in the submucosa of bronchi or bronchioles.
302
Grading of rejection of lung transplants: B3.
Expansion of the infiltrate into a dense band.
Lymphocytic satellitosis with necrosis of epithelial cells of the airway.
303
Grading of rejection of lung transplants: B4.
Severe inflammation with ulceration of the airway epithelium and fibrinopurulent exudate.
304
Alveolar adenoma: Histology.
Cystic spaces: Lined by bland type 2 pneumocytes; filled with PAS-positive granular matter.
Stroma: Rich in spindle cells; shows focal myxoid change.
305
Papillary adenoma of the lung: Histology.
True papillae lined by cuboidal or columnar cells.
Ciliated or oxyphilic cells may be present.
306
Papillary adenoma of the lung: Which features should be minimal or absent (3)?
Intracellular mucin.
Atypia.
Mitotic activity.
307
Mucous-gland adenoma:
A. Clinical presentation.
B. Histology.
A. Obstructive symptoms.
B. Bland mucin-producing cells line cysts, glands, microacini, tubules, and papillae.
308
Mucinous cystadenoma:
A. Size.
B. Histology.
A. 1-5 cm.
B. Cystic spaces lined by incomplete layer of mucinous cells; giant-cell reaction to extravasated mucin.
309
Bronchogenic squamous dysplasia or squamous-cell carcinoma in situ:
A. Clinical detection.
B. Clinical appearance.
A. Facilitated by autofluorescence bronchoscopy.
B. Flat or superficial (75%); nodular or polypoid (25%).
310
Bronchogenic squamous-cell carcinoma in situ: Typical location.
Near bifurcations in segmental bronchi.
311
Bronchogenic squamous dysplasia: Grades.
Mild, moderate, severe.
312
Bronchogenic severe squamous dysplasia vs. squamous-cell carcinoma in situ.
SCC in situ: Extreme atypia involving full thickness of epithelium.
313
Basal-cell hyperplasia of the airways: Definition.
Respiratory epithelium with more than three layers of basal cells; otherwise normal.
314
Squamous-cell carcinoma in situ vs. invasive carcinoma in a transbronchial biopsy:
SCC in situ:
− Smooth edges of the fragments.
− Flat epithelial surface.
− Smooth basement membrane.
315
Putative order of progression to bronchogenic squamous-cell carcinoma.
Basal cell hyperplasia, squamous metaplasia, squamous dysplasia, SCC in situ, invasive SCC.
316
Atypical adenomatous hyperplasia: Situation.
Almost always found incidentally in lungs with preexisting adenocarcinoma (invasive or in situ).
317
Atypical adenomatous hyperplasia: Size.
Up to 5 mm in diameter.
318
Atypical adenomatous hyperplasia: Histology (3).
Atypical pneumocytes line discrete area of thickened alveolar septa.
Gaps between cells.
Essentially no ciliated or mucous cells.
319
Atypical adenomatous hyperplasia: Immunohistochemistry.
Demonstrates surfactant protein A (PE10) in up to 25% of cases.
320
Atypical adenomatous hyperplasia vs. adenocarcinoma in situ (2).
Adenocarcinoma in situ:
− At least 5 mm (usually >10 mm) in diameter.
− May show central collapse with fibrosis or scar.
321
Atypical adenomatous hyperplasia vs. diffuse interstitial lung disease.
Diffuse interstitial lung disease: Accompanying interstitial inflammation.
322
Atypical adenomatous hyperplasia vs. peribronchiolar metaplasia.
Peribronchiolar metaplasia consists of ciliated cells.
323
Atypical adenomatous hyperplasia vs. papillary adenoma.
Atypical adenomatous hyperplasia has no true papillae.
324
Atypical adenomatous hyperplasia: Mutated genes.
KRAS and EGFR.
325
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Histology.
Increase in individual cells or in groups of neuroendocrine cells in the bronchial epithelium.
326
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Progression.
Breach of basement membrane to form carcinoid tumorlets.
327
Diffuse idiopathic pulmonary neuroendocrine cell hyperplasia: Significance.
May be the precursor of low-grade, peripheral carcinoids.
328
Carcinoid tumorlet vs. carcinoid tumor.
Carcinoid tumor: More than 5 mm in diameter.
329
Bronchogenic adenocarcinoma in situ: Former name.
Bronchioloalveolar carcinoma.
330
Bronchogenic adenocarcinoma in situ: Subtypes.
Nonmucinous (more common).
Mucinous.
331
Bronchogenic adenocarcinoma in situ: How to report on a biopsy.
As lepidic pattern (could be either adenocarcinoma in situ or invasive adenocarcinoma).
332
Bronchogenic adenocarcinoma in situ: Mutated genes.
KRAS and EGFR.
333
Bronchogenic adenocarcinoma: Frequency of metastatic disease at presentation.
About 50%.
334
Bronchogenic adenocarcinoma: Important feature of gross pathology.
Tumor is usually singular but can be multiple.
335
Minimally invasive adenocarcinoma of the lung:
A. Definition.
B. Histology.
A. Tumor is no more than 3 cm in size, with an invasive component of no more than 5 mm.
B. Mucinous or nonmucinous.
336
Bronchogenic adenocarcinoma: Overall importance of growth patterns.
Their percentages should be estimated in the report.
337
Bronchogenic adenocarcinoma in situ: Histology.
Noninvasive growth of cells along alveolar septa.
No gaps between cells.
Possible slight thickening of septa with desmoplasia.
338
Bronchogenic adenocarcinoma: Acinar pattern.
Acini and tubules resembling bronchial glands.
339
Bronchogenic adenocarcinoma: Papillary pattern.
True papillae lined by large, atypical cells.
Papillary may form secondary or tertiary branches.
There may be psammoma bodies.
340
Bronchogenic adenocarcinoma: Lepidic pattern.
Noninvasive growth along alveolar septa.
Often seen at the edge of an invasive adenocarcinoma.
341
Bronchogenic adenocarcinoma, solid pattern: Criterion for diagnosis.
At least 5 mucin droplets per 2 high-power fields.
342
Significance of specific patterns of bronchogenic adenocarcinoma:
A. Solid pattern.
B. Clear-cell pattern.
A. More likely to have the EML4−ALK mutation if there is >10% signet-cell change.
B. If extensive, must be distinguished from renal-cell carcinoma.
343
Bronchogenic adenocarcinoma: Other patterns.
Micropapillary.
Signet-ring.
344
Fetal adenocarcinoma of the lung: Histology.
Resembles fetal lung in the pseudoglandular stage; reminiscent of endometrioid adenocarcinoma.
345
Bronchogenic adenocarcinoma: Helpful immunohistochemical marker.
Napsin A is expressed by pulmonary adenocarcinomas but not by other carcinomas except those of the kidney.
346
Invasive mucinous adenocarcinoma of the lung: Immunohistochemistry.
Usually negative for TTF-1 and may also lack CK7.
347
Use of BG8 in immunohistochemistry.
As a marker of epithelial cells; not expressed by mesothelial cells.
348
Mutations of KRAS in bronchogenic adenocarcinoma:
A. Exons (3).
B. Frequent clinical association.
A. 12, 13, 61.
B. Smoking.
349
Mutations of EGFR in bronchogenic adenocarcinoma:
A. Exons.
B. Clinical associations (3).
A. 18-21.
B. Female sex; never having smoked; Asian ethnicity.
350
Bronchogenic adenocarcinoma in situ: Diagnostic criteria (5).
Three of the following:
− Stratification of cells is marked.
− Coarse chromatin with large nucleoli.
− Height of cells is increased.
− Mitotic figures.
− Overlap of nuclei due to crowding of cells.
351
Bronchogenic adenocarcinoma: Mutated genes (3).
KRAS: 30% (most common).
EGFR.
EML4−ALK.
352
Bronchogenic squamous-cell carcinoma: Variants (5).
Papillary.
Clear-cell.
Small-cell.
Basaloid.
Non-keratinizing.
353
Bronchogenic squamous-cell carcinoma: Variant associated with a better prognosis.
Papillary variant.
354
Bronchogenic squamous-cell carcinoma: Histology of the small-cell variant.
Cytoplasm: Moderately abundant; distinct intercellular boundaries.
Nuclei: Irregular; large nucleoli; no molding; no salt-and-pepper chromatin.
355
Bronchogenic squamous-cell carcinoma: Differential diagnosis of the basaloid variant.
Adenoid-cystic carcinoma:
− Younger patients.
− Better prognosis.
356
Use of p40 in the immunohistochemistry of lung carcinomas.
Positive in SCC, negative in adenocarcinoma.
357
Bronchogenic squamous-cell carcinoma: Most common mutation.
Gain of 3q26.
358
Bronchogenic squamous-cell carcinoma: Important molecular prognostic marker.
Loss of p16INK4A correlates with shorter survival.
359
Bronchogenic squamous-cell carcinoma: Molecular targets of therapy (4).
PIK3CA, SOX2, DDR2, BRF2.
360
Small-cell carcinoma of the lung: Consistent mutation.
−3p, which may cause loss of FHIT (fragile histidine triad gene).
361
Small-cell carcinoma of the lung: Less consistently mutated genes (3).
Bcl-2.
p53.
Rb.
362
Small-cell carcinoma of the lung: Importance of immunohistochemistry.
The diagnosis of small-cell carcinoma is made by light microscopy, even if all neuroendocrine markers are negative (which occurs in <10% of cases).
363
Small-cell carcinoma of the lung: Paraneoplastic syndromes (4).
Lambert-Eaton syndrome.
Cerebellar degenerative syndromes.
Cushing's syndrome.
SIADH.
364
Large-cell carcinoma: Cytology.
Nuclei: Large, vesicular; large nucleolus.
Cytoplasm: Moderately abundant; discrete cell borders.
365
Adenocarcinomas of the lung that are usually negative for TTF-1 by immunohistochemistry (3).
Mucinous adenocarcinoma.
Squamous-cell carcinoma.
Large-cell carcinoma.
366
Large-cell neuroendocrine carcinoma: Histology.
Nuclei: Large nucleolus.
Cytoplasm: Abundant.
Arrangement of cells: Nests, trabeculae, rosettes.
367
Large-cell neuroendocrine carcinoma: Immunohistochemistry (2,2).
Positive: Cytokeratin, CEA.
Variable: TTF-1.
Staining for neuroendocrine markers is often patchy and weak.
368
Pulmonary carcinoid tumor: Environmental association.
Atypical carcinoid is associated with smoking.
369
Pulmonary carcinoid tumor: Sites.
Bronchial (most tumors): May cause obstructive symptoms.
Peripheral: Usually asymptomatic.
370
Pulmonary carcinoid tumor: Stroma.
Vascular; may contain metaplastic bone or cartilage.
371
Pulmonary carcinoid tumor: Typical.
>0.5 cm in diameter.
Fewer than 2 mitotic figures per 10 hpf.
No necrosis.
372
Pulmonary carcinoid tumor: Atypical.
2-10 mitotic figures per 10 hpf.
- or -
Focal necrosis.
373
Pulmonary carcinoid tumor: Prognosis (2).
Typical: Five-year survival rate is 90%.
Atypical: Five-year survival rate is 50%.
374
Expression of neuroendocrine markers by neuroendocrine tumors.
Typical carcinoid: Strong expression.
Atypical carcinoid: Slightly weaker.
Small-cell carcinoma and large-cell neuroendocrine carcinoma: Weakest.
375
Pulmonary carcinoid tumor: Features that are not used to distinguish typical from atypical tumors (2).
Nuclear atypia and pleomorphism.
Intensity of staining for neuroendocrine markers.
376
Carcinoid tumors: Positive non-neuroendocrine markers (3).
Cytokeratin.
CD99.
S100 (sustentacular cells).
377
Pulmonary hamartoma: Typical location.
Peripheral (90%).
378
Pulmonary hamartoma: Radiography.
Fat and calcification on high-resolution CT.
379
Pulmonary hamartoma: Histology.
Nodules of mature cartilage, fat, smooth muscle, fibromyxoid tissue, and/or bone.
Benign respiratory epithelium lines the clefts that separate the nodules.
380
Pulmonary hamartoma: Associated chromosomal regions (2).
12q15 and 6p21: High-mobility-group loci.
381
Pulmonary hamartoma vs. pulmonary chondroma (2).
Pulmonary chondroma:
− No clefts between nodules.
− Often multiple.
382
Carney's triad.
Pulmonary chondromas.
Epithelioid gastrointestinal stromal tumor.
Extraadrenal paraganglioma.
383
Pulmonary lipoma: Typical site (2).
Within central bronchi.
Left side.
384
Lymphangioleiomyomatosis: Gender (2).
Sporadic: Females.
Associated with tuberous sclerosis: Both sexes.
385
Lymphangioleimyomatosis: Clinical behavior.
Destructive and metastatic; considered a low-grade tumor.
386
Lymphangioleiomyomatosis: Associated tumors (5).
Lymphangioleimyomas of the lungs.
Lymphangioleimyomas of lymph nodes.
Renal angiomyolipomas.
Hamartomas.
Uterine leiomyomas.
387
Lymphangioleimyomatosis: Clinical presentation (5).
Progressive dyspnea.
Cough.
Chylous pleural effusion.
Recurrent pneumothorax.
Hemoptysis.
388
Lymphangioleimyomatosis: Gross pathology.
Hyperaerated lungs containing many cysts, 0.5-2 cm, that distort the pleural surface.
389
Lymphangioleimyomatosis: Types of lesion (2).
Disordered proliferation of smooth-muscle cells near bronchi and vessels.
Air-filled cyst with a wall of disordered smooth muscle.
390
Lymphangioleimyomatosis: Types of cell (2).
Small spindle cells that express proliferating-cell nuclear antigen.
Larger, epithelioid cells that express HMB-45.
391
Lymphangioleimyomatosis histology score.
LHS-1: Less than 25% of the lung tissue is replaced by cysts and nodules of LAM.
LHS-2: 25% to 50%.
LHS-3: More than 50%.
392
Lymphangioleimyomatosis: Immunohistochemistry (4).
Positive: HMB-45, estrogen receptor, bcl-2; D2-40 (often).
393
Lymphangioleimyomatosis: Abnormal gene, its location, and its product.
TSC2 on 16p13 encodes tuberin.
394
Lymphangioleimyomatosis: Possible origins of pulmonary lesions.
Migration or metastasis of LAM cells from
- Angiomyolipomas.
- Lymph nodes.
395
Lymphangioleimyomatosis: Associated tumor of the central nervous system in sporadic cases.
Meningioma.
396
Inflammatory myofibroblastic tumor: Viral associations (2).
Pulmonary: HHV-8.
Splenic and nodal: EBV.
397
Inflammatory myofibroblastic tumor: Histology (4).
Fascicles or whorls of spindle cells.
Inflammatory cells.
Variable stroma.
Tumor cells may invade vessel walls and adjacent structures.
398
Inflammatory myofibroblastic tumor: Cytology (2).
Rare cells may have vesicular nuclei and large nucleoli.
Mitotic figures are usually (but not always) few.
399
Inflammatory myofibroblastic tumor: Inflammatory component.
Consists of plasma cells, lymphocytes, histiocytes, occasional Touton-type giant cells, granulocytes.
May obscure the spindle cells.
400
Inflammatory myofibroblastic tumor: Immunohistochemistry (3,3,2).
Positive: Vimentin, SMA, MSA.
Negative: S-100, CD117, myogenin.
ALK and p80 are positive in 45% of cases, more likely in the larger cells with vesicular nuclei.
401
Inflammatory myofibroblastic tumor vs. inflammatory fibrosarcoma.
Inflammatory fibrosarcoma has more nuclear atypia.
402
Inflammatory myofibroblastic tumor: Putative cell of origin.
The fibroblastic reticulum cell.
403
Pleuropulmonary blastoma: Location.
Arises in parenchyma of lung but involves pleura or mediastinum.
404
Types of pleuropulmonary blastoma: Age groups.
Type I: Infants.
Type II: Toddlers.
Type III: Pre-schoolers.
405
Type of pleuropulmonary blastoma: Relevance.
Type III has the worst prognosis.
Tumors of type I may progress to type III unless resected.
406
Types of pleuropulmonary blastoma: Gross pathology.
Type I: Purely cystic.
Type II: Solid and cystic.
Type III: Solid.
407
Pleuropulmonary blastoma: Cellular components.
Small, blue blastemal cells.
Large, spindle-shaped, often rhabdomyoblastic cells.
No epithelial cells.
408
Pleuropulmonary blastoma: Histology (3).
Sheets of blastemal cells.
Sarcomatous foci: Malignant cartilage, fat, skeletal muscle, or undifferentiated sarcoma.
Multifocal necrosis.
409
Pleuropulmonary blastoma: Possibly associated gene.
DICER1, which encodes an endoribonuclease that generates small non-encoding regulatory RNAs.
410
Pleuropulmonary blastoma vs. metastatic Wilms' tumor.
Wilms' tumor: Positive for cytokeratin (epithelial component) and WT-1.
411
Adult pulmonary blastoma: Epidemiology.
More common in females.
412
Adult pulmonary blastoma: Histology.
Biphasic, sarcomatoid carcinoma:
− Epithelium: Resembles pseudoglandular stage of fetal lung development (or endometrioid carcinoma with subnuclear or supranuclear vacuoles).
− Stroma: Blastema with occasional sarcomatous differentiation.
413
Pulmonary MALT lymphoma: Histology (4).
Centrocyte-like monomorphic cells form nodules that infiltrate alveolar septa.
Lymphoepithelial lesions involve bronchial epithelium.
Tumor cells may colonize follicles or mantle zones.
Plasma cells and plasmacytoid lymphocytes may be seen.
414
Pulmonary MALT lymphoma: Mutations (4).
Trisomy 3.
t(11;18)(q21;q21).
t(14;18)(q32;q21).
t(1;14)(q22;q32).
415
MALT lymphoma: Frequently involved gene.
JH region of immunoglobulin heavy chain.
416
Pulmonary MALT lymphoma vs. CLL: Histology.
CLL: Infiltrate does not involve the lung parenchyma.
417
Pulmonary Langerhans'-cell histiocytosis:
A. Age group.
B. Association.
A. Ages 30-50.
B. Smoking.
418
Pulmonary Langerhans'-cell histiocytosis: Difference from extrapulmonary LCH.
Pulmonary LCH is a reactive proliferation.
Extrapulmonary LCH is a neoplasm.
419
Pulmonary Langerhans'-cell histiocytosis: Radiography (2).
Early: Many subcentimeter nodules, esp. in the upper and middle lobes.
Later: Nodules are larger and have central lucency due to cavitation or bronchiolar dilatation.
420
Pulmonary Langerhans'-cell histiocytosis: Histology (4).
Stellate or Medusa-head nodules centered on bronchioles.
Nodules consist of Langerhans' cells (grooved nuclei), eosinophils, lymphocytes, plasma cells.
Nodules may invade vessels.
Progression from cellular nodules to fibrotic scars.
421
Pulmonary Langerhans'-cell histiocytosis: Associated histologic finding.
Respiratory bronchiolitis.
422
Pulmonary Langerhans'-cell histiocytosis: Expressed cytokines (2).
TGF-β₁.
GM-CSF.
423
Langerhans' cells: Immunohistochemistry.
Positive: S-100, CD1a, Langerin.
Negative: CD68.
424
Reactive eosinophilic pleuritis:
A. Cause.
B. Histology.
A. Pneumothorax.
B. Eosinophils mixed with proliferating mesothelial cells and mononuclear cells.
425
Post-transplantation lymphoproliferative disorder: Definition.
Abnormal lymphocytic proliferation (benign or malignant) occurring during immunosuppression and often involving the transplanted organ.
426
Post-transplantation lymphoproliferative disorder: Origin of proliferating cells.
Bone marrow: The donor.
Solid organs: The recipient.
427
Post-transplantation lymphoproliferative disorder: Timing.
Occurs 1 month to 4 years after the transplantation.
428
Post-transplantation lymphoproliferative disorder: Viral association.
EBV.
429
Post-transplantation lymphoproliferative disorder in the lung: Types.
Plasmacytic hyperplasia.
Polymorphic lymphoproliferative disorder.
Malignant lymphoma or multiple myeloma.
430
PTLD of the lung: Association of type with age.
Plasmacytic hyperplasia is the most common type in children and young adults.
431
PTLD of the lung: Histology of plasmacytic hyperplasia.
Proliferation of small polyclonal T and B lymphocytes, plasma cells, occasional immunoblasts.
Preserved pulmonary architecture.
432
PTLD of the lung: Histology of polymorphic lymphoproliferative disorder.
Clonal lymphocytes, plasmacytoid cells; immunoblasts that may resemble Reed-Sternberg cells.
Distorted pulmonary architecture.
433
PTLD of the lung: Most common type of lymphoma.
Diffuse large B-cell lymphoma.
434
Post-transplantation lymphoproliferative disorder: Treatment.
Reduction in immunosuppression during the first year after transplantation.
435
Malignant mesothelioma: Prognosis (2).
Death in <1 year; a few months later for epithelioid mesothelioma.
436
Malignant mesothelioma: Relation to smoking.
Smoking does not increase the risk of mesothelioma.
Smoking + asbestos = greatly increased risk for lung carcinoma.
437
Malignant mesothelioma: Variants.
Epithelioid.
Sarcomatoid.
Biphasic.
438
Epithelioid mesothelioma: Cytology.
Bland, homogeneous.
Nuclei: Round, vesicular; large nucleolus.
439
Epithelioid mesothelioma: Histology.
Grows in tubules, papillae, glands or acini, solid sheets, or a combination of patterns.
440
Desmoplastic mesothelioma:
A. Definition.
B. Frequency.
A. A collagenous subtype of sarcomatoid mesothelioma.
B. Makes up about 10% of malignant mesothelioma.
441
Biphasic mesothelioma: Criterion.
The tumor must be at least 10% epithelioid and at least 10% sarcomatoid.
442
Malignant mesothelioma: Most common variant.
Epithelioid.
443
Malignant mesothelioma: "Specific" markers (4).
Calretinin.
CK5/6.
WT-1.
D2-40.
444
Malignant mesothelioma: Nonspecific markers (2).
Pancytokeratin.
CK7.
445
Malignant mesothelioma: Mutation.
Homozygous deletion of CDKN2A/ARF at 9p21.
446
Malignant mesothelioma: Best immunohistochemical marker and its interpretation.
Calretinin:
- Stains cytoplasm of all mesothelial cells.
- Stains nuclei of malignant mesothelioma.
447
Malignant mesothelioma vs. reactive mesothelial hyperplasia: Histology.
Reactive mesothelial hyperplasia does not invade the parietal pleural fat.
448
Malignant mesothelioma vs. reactive mesothelial hyperplasia: Immunohistochemistry (4).
Reactive mesothelial hyperplasia: Cytoplasmic desmin.
Malignant mesothelioma: Linear EMA, strong p53, GLUT1.
449
Desmoplastic mesothelioma vs. chronic fibrosing pleuritis.
Chronic fibrosing pleuritis: Combinations of cytokeratins demonstrate orderly growth and absence of invasion of pleural fat.
450
Solitary fibrous tumor of the pleura: Histology (3).
Uniform spindle cells in a collagenous stroma.
Alternating hypocellular and hypercellular areas.
Hemangiopericytoma-like vascular pattern.
451